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Saturday, August 1, 2015

Hepatopulmonary Syndrome (HPS) results in the deterioration of arterial oxygenation in the setting of liver disease, which is associated with the formation of intrapulmonary vascular dilations (IPVD). The exact cause of such IPVDs is not known but may be related to an increase in vasodilators such as nitric oxide. IPVDs subsequently result in increased ventilation-perfusion mismatch, increased alveolar-arterial gradient (PA-aO2), and decreased partial arterial O2 pressure (PaO2). The only current recognized treatment for HPS is liver transplantation (LT). The authors conducted a MEDLINE literature search which detected patients 18 years or older with HPS undergoing transjugular intrahepatic portosystemic shunt (TIPS) formation for any indication from January 1990 to April 2015. The study identified 12 patients, 10 of which had either very severe or severe HPS, and all of which underwent successful TIPS placement and were followed for an average of 9.3 months. Of the 12 patients, 9 patients had improvements in oxygenation while the remaining 3 did not significantly change. After 4 months, 2 of the 9 patients with initial improvements reverted back to levels before TIPS, and 1 of the 3 patients without a change in oxygenation eventually worsened. Five of the 12 patients identified in the literature review underwent MAA shunt fraction evaluation, and 4 of which demonstrated improvements with decreased shunt fractions following TIPS. The authors conclude that the results of this literature search warrant further evaluation of TIPS in the management of HPS.

Comment:

Although the authors do acknowledge the limitations of this literature search, namely the small sample size and short follow up duration, the positive initial improvements in arterial oxygenation after TIPS in 9 of the 12 HPS patients identified certainly is encouraging. Additionally the authors underline the lack of complications reported in the series of patients, which is notable given that most of the patients had very severe HPS, which disputes the conceivable conclusion that these patients would be more susceptible to intra-procedural complications. After more investigation, TIPS may play a much larger role in the management of HPS.